Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Rheumatology > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

What is Systemic Lupus Erythematous (SLE)

A

A chronic autoimmune (seropositive) disease that can affect almost any organ system, therefore presentation can be very variable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Autoantibodies associated with SLE

A

ANA (99% sensitive, but not specific)
Anti-DNA (70% sensitive, but 99% specific)
Anti-Sm (33% sensitive, 99% specific)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

main organs/systems affected by SLE

A 
skin 
joints 
kidneys 
blood cells 
nervous system
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what type of hypersensitivity reaction is SLE

A

Type III (immune complex)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

immunological response in SLE

A

Anti-DNA antibodies and ANA antibodies form immune complexes with antigens which are deposited in small vessels in skin, joints, kidneys, resulting in complement activation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is the cause of SLE

A

largely unknown, but it is multifactorial

i.e. environmental insult in a genetically predisposed individual

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

is SLE more common in males or females?

Typical age of onset?

A

Females! >90%

age of onset 20s-30s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

constitutional symptoms in SLE

A

fever
malaise
weight loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

MSK symptoms in SLE

A

arthralgia (joint pain)
myalgia (muscle pain)
inflammatory arthritis - non-erosive
increased prevalence of AVN esp. femoral head

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Muco-cutaneous symptoms in SLE

A 
malar butterfly rash 
photosensitivity 
discoid lupus 
subacute cutaneous lupus 
oral/nasal ulceration 
Raynaud's phenomenon 
sclerodactyly 
alopecia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Renal symptoms in SLE

A

lupus nephritis

persistent proteinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Respiratory symptoms in SLE

A 
pleurisy 
pleural effusions
pneumonitis 
pulmonary embolism 
pulmonary hypertension 
interstitial lung disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Haematological symptoms in SLE

A

leukopenia
lymphopenia
anaemia
thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Neurological symptoms in SLE

A

seizures
psychosis
headache
aseptic meningitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Cardiac symptoms in SLE

A 
pericarditis 
pericardial effusion 
pulmonary hypertension 
sterile endocarditis 
accelerated ischaemic heart disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

GI symptoms in SLE

A

autoimmune hepatitis
pancreatitis
mesenteric vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Ix for SLE

A

FBC - anaemia, leukopenia, thrombocytopenia

Immunology -
ANA - sensitive, but not specific. Take seriously if other markers are also positive
Anti-DNA - specific, but only +ve in 60%

Urinalysis -
glomerulonephritis

Imaging -
evidence of organ involvement
CT, MRI, echo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

General approach to Tx of SLE

A

Depends on its manifestations

counselling, regular monitoring, avoidance of excessive sun exposure, pregnancy issues (often flares)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Tx of skin disease and arthralgia in SLE

A
  1. Hydroxychloroquine (DMARD)
  2. NSAIDs (naproxen) - for as short a period as poss
  3. Corticosteroids (pred)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Tx of SLE if there is inflammatory arthritis or organ involvement

A
  1. Immunosuppression (azothioprine)
  2. DMARDs
  3. Corticosteroids (pred)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Tx of SLE if there is lupus nephritis or CNS involvement

A
  1. Cyclophosphamide (alkylating chemo agent)

2. IV steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Tx of SLE in unresponsive cases

A

biologic therapy - Rituximab

IV immunoglobulins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

How should SLE be monitored

A

Check anti-DsDNA antibodies and complement levels regularly

Check urinalysis for blood or protein

Cardio - BP and cholesterol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is Sjogrens syndrome

A

Autoimmune seropositive condition that is characterised by lymphocytic infiltrates in exocrine glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What is an exocrine gland
glands that produce and secrete substances onto an epithelial surface by way of a duct
26
What glands are usually affected in Sjogren's syndrome
Lacrimal --> therefore dry eyes (keratonconjunctivitis sicca) salivary --> therefore dry mouth (xerostomia)
27
Apart from dry eyes and mouth, what other symptoms can be present in Sjogrens syndrome
``` arthralgia fatigue vaginal dryness parotid gland swelling peripheral neuropathy interstitial lung disease ```
28
Autoantibodies associated with Sjogrens syndrome
Anti-Ro | Anti-La
29
Does Sjogren's syndrome always occur on its own
No! Can be primary Sjogren's syndrome (no other conditions) Or secondary Sjogren's syndrome - to other autoimmune conditions e.g. RA, SLE
30
Test for ocular dryness in Sjogrens
Schirmer's test quantitative measure of tears. filter paper placed in lower conjunctival sac. test is +ve is <5mm of paper is wet after 5 mins.
31
Tx of Sjogren's syndrome
Dry eyes - luricating eye drops Dry mouth - artificial saliva, pilocarpine (cholinergic) Arthralgia and fatigue - hydroxychloroquine Severe systemic disease - immunosuppression
32
What is systemic sclerosis also known as
scleroderma
33
What is systemic sclerosis
a mulit-system seropositive autoimmune disease characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs.
34
2 main subtypes of systemic sclerosis & auto-antibodies associated with each
limited systemic sclerosis - anti-centromere antibody diffuse systemic sclerosis - anti Scl-70 antibody
35
What is the difference between limited and diffuse systemic sclerosis
more rapid development of skin changes and earlier organ involvement in diffuse systemic sclerosis compared to limited systemic sclerosis
36
cutaneous presentations of systemic sclerosis
(skin eventually becomes thickened and tightened) RAYNAUDS!! centrally located skin sclerosis on arms, faces and/or neck scerodactyly (thickening of skin in fingers and toes) telangiectasia (red dots on the fingers, palms, face) calcinosis (subcutaneous deposits of calcium in digits)
37
organ presentations of systemic sclerosis
``` pulmonary hypertension pulmonary fibrosis accelerated hypertension eventual renal crisis inflammatory arthritis myositis gut involvement - presents as dysphagia, malabsorption, bacterial overgrowth ```
38
pneumonic to remember presentation of limited systemic sclerosis
CREST syndrome ``` Calcinosis Raynaud's Esophageal & gut dysmotility Sclerodactyly Telangiectasia ```
39
Mx of renal crisis in scleroderma
1. ACEi - lisinopril 2. additional anti-hypertensives 3. renal dialysis or transplant
40
Mx of raynaud's in scleroderma with no digital ulceration
1. CCB - amlodipine, nifedipine | 2. aspiriin or Pentoxifylline
41
Mx of Raynauds in scleroderma with digital ulceration
1. phosphodiesterase 5 inhibitor - sildenafil, bostentan | 2. Iloprost
42
Mx of skin involvement in scleroderma
1. topical immollient pruritus - steroids thickening - cyclophosphamide
43
Mx of GI involvement in scleroderma
PPI - omeprazole
44
Mx of interstitial lung disease in scleroderma
cyclophosphamide
45
what is Mixed Connective Tissue Disease (MCTD)
an overlap syndrome - a group of conditions that meets the classification criteria for more than 1 well recognised rheumatological disease
46
Features of MCTD
``` Raynauds Arthralgia/arthritis Myositis Sclerodactyly Pulmonary hypertension Interstitial lung disease ```
47
Auto-antibody associated with MCTD
Anti-RNP antibodies
48
What is anti-phospholipid syndrome
an autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss.
49
auto-antibodies associated with anti-phospholipid syndrome
Anticardiolipin antibodies | Lupus anticoagulant antibodies
50
pneumonic to remember anti-phospholipid syndrome
CLOT Coagulation defect Livedo reticularis Obstetric (recurrent miscarriage) Thrombocytopenia
51
What is the criteria for pregnancy loss in anti-phospholipid syndrome
Pregnancy loss with no other explanation 10-34/40 weeks or 3 pregnancy losses with no other explanation <10/40 weeks
52
What is livedo reticularis
Lace-like purplish discolouration of skin seen in anti-phospholipid syndrome
53
What valvular heart problem can be found in patients with Anti-Phospholipid syndrome
Sterile endocarditis --> Libman-Sacks endocarditis embolisation can occur from these lesions
54
Tx of anti-phospholipid syndrome
initial anti-coagulation in acute thrombosis --> LMWH then life-long warfarin pregnancy - LMWH
55
do all patients with anti-phospholipid syndrome receive anti-coagulation
No - if they have it but have never had thrombosis, they don't require anti-coagulation
56
What is gout
a crystal arthropathy caused by deposition of URATE crystals within a joint
57
what is uric acid
the final compound in the breakdown of purines in DNA metabolism (adenine and guanine).
58
How does hyperuricaemia occur to cause gout
increased urate production e.g. inherited enzyme defects psoriasis alcohol ``` decreased renal urate excretion e.g. chronic renal impairment volume depletion e.g. heart failure hypothyroidism thiazide diuretics ```
59
most common joints affected by gout
first MTP joint ankle knee
60
presentation of gout
intense red hot swollen joint | symptoms last for 7-10 days if untreated then resolve
61
what are 'gouty tophi'
painless white accumulation of uric acid which can occur in the soft tissues and occasionally erupt through the skin
62
Ix for gout
synovial fluid sample for polarising microscopy - needle shaped crystal s - negative birefringence
63
Tx acute gout
1. NSAID 2. Colchicine 3. Corticosteroids
64
Prophylaxis for gout
Allopurinol (xanthine oxidase inhibitor)
65
What medication should NOT be given in acute gout
Allopurinol - can potentiate further flares
66
When should prophylaxis for gout be started
2 weeks after acute attack
67
Adverse effects of allopurinol
rash | azothioprine interaction
68
What is pseudogout
a crystal arthropathy caused by deposition of CALCIUM PYROPHOSPHATE crystals in a joint
69
What conditions are associated with pseudogout
hyperparathyroidism | amyloidosis
70
Ix of pseudogout
synovial fluid sample for polarising microscopy - rhomboid shaped crystals - positive birefringement
71
What is chondrocalcinosis
calcium pyrophosphate deposition in cartilage in the absence of acute inflammation
72
Tx of pseudogout
1. NSAIDs | 2. Intra-articular steroids
73
Prophylaxis for pseudogout
There is none!!
74
low levels of which complement are associated with developing SLE
C3 & C4 (C4a and C4b)
75
Drugs causing drug-induced lupus
procainamide (Na channel blocker) | hydralazine (anti-hypertensive)
76
presentation of drug induced lupus
arthralgia myalgia skin (malar rash) and pulmonary involvement (pleurisy) ANA +ve in 100%, Anti-DNA -ve
77
radiological features of gout
joint effusion (early sign) well-defined "punched out" lesions with sclerotic margins preservation of joint space (not in late) no periarticular osteopenia soft tissue tophi
78
pathogenesis of SLE
cellular remnants containing nuclear material are transferred to lymphatic tissues. They are presented to T cells which then stimulates B cells to produce autoantibodies. IgG antibodies attack DNA resulting in antigen-antibody complexes causing damage in various areas
79
pneumonic to remember the causes of gout
DART Diuretics Alcohol Renal disease Trauma

Rheumatology (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions